Studies On Pathological Changes Of The Skin In Amyotrophic Laterial Sclerosis

Amyotrophic lateral sclerosis(ALS)is a sever neurodegenerative disease characterized by progressive selective degeneration of motor neurons,the etiology and pathogenesis of which remains unknown.Because of lack of effective treatment,patients with ALS only can survive with the disease for generally 3-5 years.What`s more,the clinical diagnosis is mainly based on typical clinical manifestations and electrophysiological changes because of a lack of objective diagnostic criteria.Therefore,it`s important to explore the pathogenesis and find an objective biological indication.It`s difficult to obtain the brain or spinal cord of the ALS patients,and till now we don`t find biomarkers in the blood or cerebrospinal.In contrast,skin tissue is easy to get,moreover,during embryonic period,skin and nerve tissue both were originated from the ectoderm,so some nervous system diseases(including ALS)are accompanied by skin changes,and even more,these changes may appear earlier than the symptoms of the nervous system.Therefore,study on skin(including fibroblast)may helpful for the diagnosis of ALS and provide clues to the pathogenesis of ALS.Recently,researchers found the skin tissue of ALS exist unique changes,including:(1)both the number and diameter of collagen fibre decrease,and the content of non crystal components increase;(2)the content of ALS related protein(including FUS,TDP-43,VCP)and SOD1 protein karyoplasmic ratio increase;(3)the quantity of neurotrophic factors,such as VEGF,IGF-1,and CNTF increase;(4)the expression of inflammatory markers(including IL-6,TNF-?)increase;(5)the activity of MMPs raises.